Felty's syndrome- Infections in rheumatoid arthritis

Felty's syndrome is characterised by triad of rheumatoid arthritis, splenomegaly and neutropenia. There is a risk of recurrent infections due to neutropenia. Patient may also have anemia and thrombocytopenia because of hypersplenism. Best treatment for Felty's syndrome is to control underlying rheumatoid disease.

Patient with deforming RA found to have splenomegaly

Moyamoya disease- An uncommon cause of stroke

Moyamoya disease is a progressive, occlusive disease of the cerebral vessels with particular involvement of the circle of Willis and the arteries that form it. Collateral vessels develops around the blocked artery to compensate for the blockage, but the collateral vessels are small, weak, and prone to hemorrhage, aneurysm and thrombosis. On conventional X-ray angiography, these collateral vessels have the appearance of a "puff of smoke" for which it is named as Moyamoya (Moyamoya is term for puff of smoke in japanese). It can present as migraine type headache, recurrent transient ischemic attacks (TIAs), stroke or hemorrhage in the brain. Therapy is primarily directed at the complications of the disease.

Rich collaterals around the blocked artery giving the appearance of puff of smoke.

Gianotti-crosti syndrome : An atypical presentation of Hepatitis B virus infection

In children, Hepatitis B may present rarely with anicteric hepatitis, a non-pruritic papular rash on the face, buttocks and limbs, and lymphadenopathy. This is known as papular acrodermatitis of childhood or Gianotti- Crosti syndrome.  Likely pathogenesis for this condition is local type IV hypersensitivity reaction to offending viral antigen within the dermis. It is more common among children with atopic dermatitis. With the advent of global HBV vaccination, EBV is now more common pathogen for this uncommon disorder. It is a benign self limited condition which requires no treatment.

Child with acute hepatitis B infection with non pruritic papular rash

Lump in epigastrium

57 years old male chronic alcoholic for last 30 years presented with c/o pain abdomen, vomiting for last 20 days. Pain abdomen was constant dull aching mainly in epigastric region. Vomiting used to occur just after intake of meal and it did not contain altered or fresh blood. He also had 1 episode of melena 15 days back. Similar type of abdominal pain was present 1 year back for which he required hospitalisation for 4 days. On examination there was pallor, palpable diffuse lump in the epigastrium. There was no lymphadenopathy, icterus, cyanosis, hepatosplenomegaly. On evaluation he was found to have anemia (Hb-7.2), large pseudocyst (22 cms by 10 cms) in pancreas compressing the stomach from posterior side as seen in UGI endoscopy. There was also a duodenal ulcer in D1. A diagnosis of acute on chronic pancreatitis complicated by pseudocyst formation, etiology being alcohol, was made. For duodenal ulcer he we started on proton pump inhibitors in double strength. He also received i/v fluids, analgesics and anti-emetics. Liver function and renal function tests were normal. CECT abdomen showed hyperdense content in the pancreatic pseudocyst causing cyst in cyst appearance so possibility of pseudoaneurysmal bleed was kept, but CT angiogram did not reveal any sign of pseudoaneurysm. He received 3 units of blood transfusion in the hospital following which his haemoglobin raised from 7.2 to 9.7. Then cystogastrostomy was done for the drainage of large cyst which was eminent to rupture. Patient recovered uneventfully thereafter.

TAKE HOME MESSAGE-

1.  Most common cause of acute pancreatitis is Gall stone and of chronic pancreatitis is alcohol.

2.  Incidence of pseudocyst in acute pancreatitis varies from 5% to 20%.

3.  Pseudoaneurysm formation and subsequent rupture is one of the fearsome complication.

4.  Endoscopic drainage is effective method of management of pancreatic pseudocyst. Most commonly used technique is gastrocystostomy which is performed endoscopically.

Pain in right upper quadrant of abdomen in an elderly lady

This 60 years old lady presented with history of pain in right hypochondrium and jaundice for 5 days in the gastroenterology department. In the background history, patient was operated for gall stone disease 3 months back and surgery done was open cholecystectomy with bile duct exploration. After that ERCP was done twice to remove bile duct stones.  After the surgery patient remained asymptomatic until 5 days back when she developed pain in right hypochondrium which was moderate in intensity, continuous, non colicky, sharp piercing type of pain associated with yellowish discolouration of eyes. Jaundice was associated with generalized itching, not hampering her activities or sleep, clay coloured stools and high coloured urine. There was no h/o fever, abdominal distension, hematemesis, melena, loose motions or constipation. There was no h/o altered sensorium, decreased urine output, cough with expectoration. On examination she was found to have shock, pallor, icterus. There was no lymphadenopathy, cyanosis, edema and jvp was not raised. Her TLC was normal but renal function were deranged. Total bilirubin (Bil-14.6, Direct-12.1) was raised. So considering the possibility of cholangitis causing septic shock and acute kidney injury due to hypotension or ATN, she was started on broad spectrum antibiotics (Inj. Tazact and metrogyl) after sending the blood cultures. After correction of dehydration, she was started on inoptrope. Next day, she was taken up for ERCP, where papillotomy was done following that there was discharge of pus from the papilla and 17F by 10 cms biliary stent was placed. After that she was shifted to ward. Next day in the morning patient became drowsy, urea creatinine were found to be raised. Iv fluids were given. Antibiotics upgraded to meronem emperically as blood culture came out to be sterile. Central line was put and cvp was found to be 20 cms. ABG analysis revealed the presence of metabolic acidosis. So she was taken up for hemodialysis. After hemodialysis, she again developed hypotension, for which inotropes were upgraded and patient was shifted to icu. She remained in ICU for 4 days received hemodialysis daily along with broad spectrum antibiotics. After that, her sensorium improved and spontaneous eye opening was present and was able to follow commands with movements of limbs. Subsequently she was shifted to ward. And after 15 days she could be discharged in hemodynamically stable condition.

TAKE HOME MESSAGE-

1.   Characterstic triad of cholangitis (Charcot’s) is present only in 50 to 70 %  and pentad (Reynold's) only in 20% of cases. So a high index of suspicion in required for the diagnosis.

2. Fever although present in 90% of acute cholangitis patients, may be absent in elderly, immunocompromised and diabetic patients.

3. Most common cause of Cholangitis is choledocholithiasis.

4. Key to successful management is early institution of broad spectrum antibiotics covering gram negative (Most common offender being E. coli overall) and anaerobes, f/b drainage of pus either externally (percutaneous drainage with pigtail catheter) or internally (ERCP and stent placement)

5.   Acute cholangitis can lead to multiple organ failures quickly as seen in our case which has grave prognosis and requires supportive treatment as well.

Any comment regarding the case or management is welcomed.